Vascular rings--presentation, investigation and outcome

Our aim was to determine the presentation of patients with vascular rings and evaluate the effectiveness of investigations. Surgical outcomes and respiratory sequelae were also examined. The design was a retrospective case note study over a 13-year period set in a tertiary children's hospital. Children below the age of 16 years presenting with a vascular ring to the Royal Hospital for Sick Children, Glasgow were studied. Demographic data at presentation, including symptoms, were recorded. The ability of diagnostic investigations to identify the presence of a vascular ring was evaluated. Surgical outcomes were determined by measuring surgical complications and mortality. Respiratory sequelae were recorded by the presence of persistent symptoms or the need for tracheostomy or long-term ventilation following surgery. A total of 24 patients were identified with a median age at presentation of 4.5 months. Stridor was the commonest presenting symptom (14/24). Angiography, chest CT scanning and MRI were the most accurate imaging modalities (accurate in 100% of cases used). Chest X-ray films and echocardiography had the lowest detection rates. Surgical complications (4/24) and mortality (1/24) were low. A substantial number of patients available to follow-up (7/20) were still experiencing stridor 3 months post-operatively.

Vascular rings are rare, however, often present with common symptoms. Most children present in early infancy, but a minority presents much later. The investigation of choice is a barium swallow followed by high-resolution computed tomography. Surgery is safe although a number of patients will have persisting symptoms.